نتایج جستجو برای: mitochondriopathy

تعداد نتایج: 62  

2014
Marisa Brum Cristina Semedo Rui Guerreiro José Pinto Marques

Background. The mutation 9185T>C in ATP6 gene, associated with Leigh syndrome, was reported in only few families. Motor neuron disease (MND), both clinically and electrophysiologically, was not previously described in association with this mutation. Case Report. 33-year-old male, with family history of mitochondrial disease, presented with cognitive impairment, exercise intolerance, and progres...

Journal: :American journal of physiology. Cell physiology 2016
Stéphanie Paradis Anne-Laure Charles Alain Meyer Anne Lejay James W Scholey Nabil Chakfé Joffrey Zoll Bernard Geny

Peripheral artery disease (PAD) is a common circulatory disorder of the lower limb arteries that reduces functional capacity and quality of life of patients. Despite relatively effective available treatments, PAD is a serious public health issue associated with significant morbidity and mortality. Ischemia-reperfusion (I/R) cycles during PAD are responsible for insufficient oxygen supply, mitoc...

Journal: :Pediatric gastroenterology, hepatology & nutrition 2015
Ajay Kaul Kanwar K Kaul

Cyclic vomiting syndrome (CVS) is a functional disorder characterized by stereotypical episodes of intense vomiting separated by weeks to months. Although it can occur at any age, the most common age at presentation is 3-7 years. There is no gender predominance. The precise pathophysiology of CVS is not known but a strong association with migraine headaches, in the patient as well as the mother...

Journal: :Journal of the American Society of Nephrology : JASN 2007
Francesca Diomedi-Camassei Silvia Di Giandomenico Filippo M Santorelli Gianluca Caridi Fiorella Piemonte Giovanni Montini Gian Marco Ghiggeri Luisa Murer Laura Barisoni Anna Pastore Andrea Onetti Muda Maria Luisa Valente Enrico Bertini Francesco Emma

Primary coenzyme Q(10) (CoQ(10)) deficiency includes a group of rare autosomal recessive disorders primarily characterized by neurological and muscular symptoms. Rarely, glomerular involvement has been reported. The COQ2 gene encodes the para-hydroxybenzoate-polyprenyl-transferase enzyme of the CoQ(10) synthesis pathway. We identified two patients with early-onset glomerular lesions that harbor...

Journal: :American journal of physiology. Heart and circulatory physiology 2005
António Ascensão José Magalhães José M C Soares Rita Ferreira Maria J Neuparth Franklim Marques Paulo J Oliveira José A Duarte

The objective of this work was to test the hypothesis that endurance training may be protective against in vivo doxorubicin (DOX)-induced cardiomyopathy through mitochondria-mediated mechanisms. Forty adult (6-8 wk old) male Wistar rats were randomly divided into four groups (n = 10/group): nontrained, nontrained + DOX treatment (20 mg/kg), trained (14 wk of endurance treadmill running, 60-90 m...

2012
Alain Martelli Lisa S. Friedman Laurence Reutenauer Nadia Messaddeq Susan L. Perlman David R. Lynch Kathrin Fedosov Jörg B. Schulz Massimo Pandolfo Hélène Puccio

Friedreich's ataxia (FRDA) is the most common hereditary ataxia in the caucasian population and is characterized by a mixed spinocerebellar and sensory ataxia, hypertrophic cardiomyopathy and increased incidence of diabetes. FRDA is caused by impaired expression of the FXN gene coding for the mitochondrial protein frataxin. During the past ten years, the development of mouse models of FRDA has ...

Journal: :Journal of Korean Medical Science 1993
K. C. Jung N. H. Myong J. G. Chi H. R. Choi H. S. Lee Y. M. Ahn

Leigh's disease is a rare progressive neurological disorder that is characterized light microscopically by focal spongy necrosis in the brain and electron microscopically by mitochondriopathy. We report an autopsy case of Leigh's disease that showed abnormalities in the liver, kidney and skeletal muscle as well as the central nervous system. The patient was an 18-month-old girl who has carried ...

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